Management of von Willebrand disease: a survey on current clinical practice from the haemophilia centres of North America

The optimal treatment of patients with von Willebrand's disease (vWD) remai ns to be defined. Moreover, it has not been firmly established which, if an y, commonly measured parameters of von Willebrand factor (vWF) protein in t he plasma are useful in guiding treatment. To better understand what guidel ines physicians follow in the management of vWD, we surveyed 194 North Amer ican physicians who are members of the Hemophilia Research Society. Ninety- nine per cent of responding physicians depend on factor VIII (FVIII):C, vWF :RCo activity and vWF:AG to diagnose vWD, while only 49% use the bleeding t ime. The minimal goals of treatment for patients undergoing major surgery/t rauma or central nervous system haemorrhage were FVIII:C and vWF:RCo activi ty greater than 80% while levels of more than 50% for minor surgery and den tal extractions were considered adequate. Treatment of vWD was based on the type of vWD with type 1 patients being treated most often with desmopressi n acetate (DDAVP) alone, types 2A and 2B patients with a combination of DDA VP and a vWF-containing FVIII product, type 3 patients with vWF-containing concentrate. Viral infections, including human immunodeficiency virus, hepa titis A, B and C viruses, and parvovirus have been seen in vWD and the effi cacy of viral attenuation processes is a major criterion for the selection of treatment by physicians. Based on this survey, prospective studies need to be designed to address the clinical efficacy, safety and predictive valu e of laboratory monitoring of patients with vWD.